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. 2022 Aug 16;13:911385. doi: 10.3389/fimmu.2022.911385

Table 2.

Therapeutic agents and their targets and clinical use for AIC in PIDs.

THERAPEUTIC AGENT MOLECULAR TARGET CELLULAR TARGET CLINICAL USE
MMF IMPDH T and B cells AICs, many (25, 26)
Rituximab CD20 mature B cells AICs, many (2730)
Bortezomib proteasome plasma cells AICs, MM (3138)
Daratumumab CD38 plasma cells and plasma blasts refractory AICs, MM (3943)
Isatuximab CD38 Plasma cells and plasma blasts refractory AICs, MM (44, 45)
Belimumab BAFF B cells AIC in SLE (46, 47)
Atacicept BAFF and APRIL B cells SLE (48)
Ibrutinib BTK B cells AIC in ALL (49, 50)
Epratuzumab CD22 B cells SLE (51)
Sirolimus/Everolimus mTOR T cells ALPS, CTLA4 def, APDS, others (5255)
Abatacept CTLA4 T cells CTLA4 def (56, 57), LRBA def (58)
Leniolisib p110delta T cells APDS1 (59)
Seletalisib p110delta T cells APDS1 and APDS2 (60)
Tofacitinib JAK1, JAK3 T cells and inflammasome JAK/STAT diseases (6164)
Ruxolitinib JAK1, JAK2 T cells and inflammasome SAVI, STAT1 GOF, STAT3 GOF (61, 62)
Baricitinib JAK1, JAK2 T cells and inflammasome JAK/STAT diseases (6164)
Filgotinib JAK1 T cells and inflammasome JAK/STAT diseases (6164)
Decernotinib JAK3 T cells and inflammasome JAK/STAT diseases (6164)
Anakinra IL-1R T and B cells WAS (65), MIS-C (66, 67)
Etanercept TNF macrophages DADA2 (68)
Infliximab TNF macrophages DADA2 (68)
Adalimumab TNF macrophages DADA2 (68)
Plexirafor CXCR4 neutrophils WHIM (69, 70)
Mavorixafor CXCR4 neutrophils WHIM (71)
Eculizumab C5 complement Rescue therapy of refractory AIHA (72, 73)

AIC, autoimmune cytopenia; ALL, acute lymphatic leukaemia; ALPS, autoimmune lymphoproliferative syndrome; APDS, Activated PI3K-kinase Delta Syndrome; DADA2, deficiency od adenosine deaminase; MIS-C, multisystem inflammatory syndrome in children; MM, multiple myeloma; MMF, mycophenolate mophetile; SAVI, STING-associated vasculopathy with onset in infancy; SLE, systemic lupus erythematosus; TNF, tumor necrosis factor; WHIM, Warts, hypogammaglobulinemia, infections, and myelokathexis; WAS, Wiskott-Aldrich Syndrome.